We use cookies to understand how you use our site and to improve your experience. This includes personalizing content and advertising. To learn more, click here. By continuing to use our site, you accept our use of cookies. Cookie Policy.

Features Partner Sites Information LinkXpress
Sign In
Advertise with Us
RANDOX LABORATORIES

Download Mobile App




Novel Copper Therapy Cures ALS Mouse Model

By LabMedica International staff writers
Posted on 09 Feb 2016
Print article
Image: Copper, zinc superoxide dismutase (Cu Zn SOD) is essential to life, but when damaged can become toxic (Photo courtesy of Oregon State University).
Image: Copper, zinc superoxide dismutase (Cu Zn SOD) is essential to life, but when damaged can become toxic (Photo courtesy of Oregon State University).
Selective delivery of copper (Cu) to the central nervous system effectively treats the motor neuron disease amyotrophic lateral sclerosis (ALS) in the most widely used mouse model of the disorder.

ALS is a progressive and fatal neurodegenerative disease caused by the death and deterioration of motor neurons in the spinal cord that is linked to mutations in the enzyme copper, zinc superoxide dismutase (Cu, Zn SOD). Copper helps to stabilize the three-dimensional structure of this antioxidant enzyme, but when it lacks metal co-factors, SOD can unfold and become toxic, leading to the death of motor neurons.

Over-expression of mutant Cu, Zn SOD in mice induces ALS and has become the most widely used model of neurodegeneration. However, no pharmaceutical agent in 20 years has extended lifespan of these animals by more than a few weeks.

The Copper-Chaperone-for-SOD (CCS) protein completes the maturation of SOD by inserting copper, but paradoxically mice that have been genetically engineered to express human CCS while co-expressing mutant SOD die within two weeks of birth.

Hypothesizing that co-expression of CCS created copper deficiency in the spinal cord, investigators at Oregon State University (Corvallis, USA) treated these baby mice with the PET (positron emission tomography)-imaging agent CuATSM (diacetyl-bis(N4-methylthiosemicarbazone)), which is known to deliver copper into the central nervous system within minutes.

The investigators reported in the January 27, 2016, online edition of the journal Neurobiology of Disease that CuATSM prevented the early mortality of the CCSxSOD mice, while markedly increasing the level of Cu, Zn SOD protein in their ventral spinal cords. Remarkably, continued treatment with CuATSM extended the survival of these mice by an average of 18 months. When CuATSM treatment was stopped, these mice developed ALS-related symptoms and died within three months. Restoring CuATSM treatment could rescue these mice after they became symptomatic, providing a means to start and stop disease progression.

All human ALS patients express CCS, raising the hope that familial ALS patients could respond to CuATSM treatment similarly to the CCSxSOD mice.

"We are shocked at how well this treatment can stop the progression of ALS," said senior author Dr. Joseph Beckman, professor of biochemistry and at Oregon State University. "We have a solid understanding of why the treatment works in the mice, and we predict it should work in both familial and possibly sporadic human patients, but we will not know until we try. We want people to understand that we are moving to human trials as quickly as we can. In humans who develop ALS, the average time from onset to death is only three to four years."

Related Links:

Oregon State University


Platinum Member
COVID-19 Rapid Test
OSOM COVID-19 Antigen Rapid Test
Magnetic Bead Separation Modules
MAG and HEATMAG
Anti-Cyclic Citrullinated Peptide Test
GPP-100 Anti-CCP Kit
Gold Member
Systemic Autoimmune Testing Assay
BioPlex 2200 ANA Screen with MDSS

Print article

Channels

Clinical Chemistry

view channel
Image: The 3D printed miniature ionizer is a key component of a mass spectrometer (Photo courtesy of MIT)

3D Printed Point-Of-Care Mass Spectrometer Outperforms State-Of-The-Art Models

Mass spectrometry is a precise technique for identifying the chemical components of a sample and has significant potential for monitoring chronic illness health states, such as measuring hormone levels... Read more

Hematology

view channel
Image: The CAPILLARYS 3 DBS devices have received U.S. FDA 510(k) clearance (Photo courtesy of Sebia)

Next Generation Instrument Screens for Hemoglobin Disorders in Newborns

Hemoglobinopathies, the most widespread inherited conditions globally, affect about 7% of the population as carriers, with 2.7% of newborns being born with these conditions. The spectrum of clinical manifestations... Read more

Immunology

view channel
Image: A false color scanning election micrograph of lung cancer cells grown in culture (Photo courtesy of Anne Weston)

AI Tool Precisely Matches Cancer Drugs to Patients Using Information from Each Tumor Cell

Current strategies for matching cancer patients with specific treatments often depend on bulk sequencing of tumor DNA and RNA, which provides an average profile from all cells within a tumor sample.... Read more

Microbiology

view channel
Image: Microscope image showing human colorectal cancer tumor with Fusobacterium nucleatum stained in a red-purple color (Photo courtesy of Fred Hutch Cancer Center)

Mouth Bacteria Test Could Predict Colon Cancer Progression

Colon cancer, a relatively common but challenging disease to diagnose, requires confirmation through a colonoscopy or surgery. Recently, there has been a worrying increase in colon cancer rates among younger... Read more

Pathology

view channel
Image: Fingertip blood sample collection on the Babson Handwarmer (Photo courtesy of Babson Diagnostics)

Unique Hand-Warming Technology Supports High-Quality Fingertip Blood Sample Collection

Warming the hand is an effective way to facilitate blood collection from a fingertip, yet off-the-shelf solutions often do not fulfill laboratory requirements. Now, a unique hand-warming technology has... Read more
Copyright © 2000-2024 Globetech Media. All rights reserved.